Skip to main content

Table 1 Classification of interstitial lung diseases

From: Interstitial lung diseases in the hospitalized patient

Classification Clinical-radiological-pathological diagnosis Histopathologic pattern
ILDs of known cause Environmental or drug related Depends on underlying disease
Connective tissue disease related Depends on underlying disease
Hypersensitivity pneumonitis  
Idiopathic interstitial pneumonias (IIPs)   
 Major IIPs   
  Chronic fibrosing IPs IPF UIP
Idiopathic NSIP NSIP
  Smoking-related IPs DIP DIP
RB-ILD Respiratory bronchiolitis
  Acute/subacute IPs COP Organizing pneumonia
AIP Diffuse alveolar damage
 Rare IIPs Idiopathic LIP LIP
Idiopathic PPFE Elastotic fibrosis of pleura and subpleural parenchyma
 Unclassifiable IIPs   
Granulomatous lung disorders Sarcoidosis Non-necrotizing granuloma
Other forms of ILD LAM Cysts and proliferation of LAM cells
PLCH Proliferation of Langerhans cells
Eosinophilic pneumonia Depends on disease onset
  1. AIP acute interstitial pneumonia, COP cryptogenic organizing pneumonia, DIP desquamative interstitial pneumonia, IP interstitial pneumonia, IPF idiopathic pulmonary fibrosis, LAM lymphagioleiomyomatosis, LIP lymphocytic interstitial pneumonia, NSIP nonspecific interstitial pneumonia, PLCH pulmonary Langerhans cell histiocytosis, PPFE pleuroparenchymalfibroelastosis, RB-ILD respiratory bronchiolitis-associated interstitial lung disease, UIP usual interstitial pneumonia